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Huntington’s Disease: Neuropathological Grading

  • Jean-Paul Vonsattel
  • Richard H. Myers
  • Thomas J. Stevens
  • Robert J Ferrante
  • Peter A. Paskevich
  • Edward P. RichardsonJr.
  • Edward D. Bird
Part of the Advances in Behavioral Biology book series (ABBI, volume 32)

Abstract

The striatum was found to exhibit marked variation in the severity of neuropathological involvement in post mortem brain specimens from 163 clinically diagnosed cases of Huntington’s disease (HD). A grading system was established by macroscopic and microscopic criteria, resulting in five grades (0–4) designated in ascending order of severity. The earliest changes were seen in the medial caudate nucleus (CN), in the tail of the CN, and in the dorsal part of the putamen. The grade correlated with the extent of clinical disability as assessed by a rating scale. Quantitative measurements revealed a 50% neuronal loss in the CN in grade 1 and a 95% loss in grade 4. Astrocytes are greatly increased in grades 2–4. Analyses of the CN in grade 4 reflect mainly astrocytic composition with a component of remote neurons projecting to the striatum. Because of the relative preservation of the lateral half of the head of the CN in grades 1–2, these regions would reflect early cellular and biochemical changes in HD.

Keywords

Nucleus Accumbens Neuronal Loss Caudate Nucleus Globus Pallidus Brodmann Area 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Plenum Press, New York 1987

Authors and Affiliations

  • Jean-Paul Vonsattel
    • 1
    • 2
    • 3
  • Richard H. Myers
    • 4
  • Thomas J. Stevens
    • 3
  • Robert J Ferrante
    • 1
    • 2
  • Peter A. Paskevich
    • 5
  • Edward P. RichardsonJr.
    • 1
    • 2
  • Edward D. Bird
    • 3
  1. 1.C.S. Kubik Laboratory for Neuropathology, James Homer Wright Pathology LaboratoriesMassachusetts General HospitalUSA
  2. 2.Department of Neurology-NeuropathologyHarvard Medical SchoolBostonUSA
  3. 3.Ralph Lowell Laboratories, Mailman Research CenterMcLean Hospital and Harvard Medical SchoolBelmontUSA
  4. 4.Department of NeurologyBoston University Medical School and Massachusetts General Hospital, Harvard Medical SchoolBostonUSA
  5. 5.Laboratories for Psychiatric Research, Mailman Research CenterMcLean Hospital and Harvard Medical SchoolBelmontUSA

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